ENDOCRINOLOGY AND METABOLISM: Edited by Sally RadovickUpdate on adrenarcheWitchel, Selma Feldmana; Pinto, Biancaa; Burghard, Anne Claireb; Oberfield, Sharon E.b Author Information aDivision of Pediatric Endocrinology, UPMC Children's Hospital of Pittsburgh, Pittsburgh, Pennsylvania bDivision of Pediatric Endocrinology, Columbia University Irving Medical Center-Morgan Stanley Children's Hospital, New York, New York, USA Correspondence to Selma Feldman Witchel, MD, Division of Pediatric Endocrinology, Department of Pediatrics, UPMC Children's Hospital of Pittsburgh, University of Pittsburgh, 4401 Penn Avenue, Pittsburgh, PA 15224, USA. Tel: +1 412 692 5170; fax: +1 412 692 5834; e-mail: [email protected] Current Opinion in Pediatrics 32(4):p 574-581, August 2020. | DOI: 10.1097/MOP.0000000000000928 Buy Metrics Abstract Purpose of review Adrenarche is the pubertal maturation of the innermost zone of the adrenal cortex, the zona reticularis. The onset of adrenarche occurs between 6 and 8 years of age when dehydroepiandrosterone sulfate (DHEAS) concentrations increase. This review provides an update on adrenal steroidogenesis and the differential diagnosis of premature development of pubic hair. Recent findings The complexity of adrenal steroidogenesis has increased with recognition of the alternative ‘backdoor pathway’ and the 11-oxo-androgens pathways. Traditionally, sulfated steroids such as DHEAS have been considered to be inactive metabolites. Recent data suggest that intracellular sulfated steroids may function as tissue-specific intracrine hormones particularly in the tissues expressing steroid sulfatases such as ovaries, testes, and placenta. Summary The physiologic mechanisms governing the onset of adrenarche remain unclear. To date, no validated regulatory feedback mechanism has been identified for adrenal C19 steroid secretion. Available data indicate that for most children, premature adrenarche is a benign variation of development and a diagnosis of exclusion. Patients with premature adrenarche tend to have higher BMI values. Yet, despite greater knowledge about C19 steroids and zona reticularis function, much remains to be learned about adrenarche. Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.